Background
The native liver of patients with maple syrup urine disease (MSUD) (1st recipients) can be used as a graft for non‐MSUD patients with end‐stage liver disease (2nd recipients). This study aimed to demonstrate the optimal operational procedures and the long‐term outcomes of 2nd recipients.
Methods
Six 2nd recipients of living donor domino liver transplantation (LD‐DLT) (age: 42.5 [22–169] months at DLT) received a native liver as a graft from an MSUD patient at our hospital between June 2014 and April 2020. We reviewed the operational procedures and outcomes of 2nd recipients after LD‐DLT.
Results
The 2nd recipients' original diseases included biliary atresia, congenital hepatic fibrosis, congenital protein C deficiency, familial hypercholesterolemia, hepatoblastoma, and mitochondrial hepatopathy. Five of the six recipients had a whole liver and one had a right lobe graft. The site at which the vessels of the MSUD liver were dissected prioritized the safety of the 1st recipient. At the end of follow‐up, all recipients were doing well without surgical complications. The mean serum amino acid values of the 2nd recipients did not exceed the upper limit of the reference values during the long‐term observation period. All patients showed normal growth while maintaining the same z‐score of height and weight after LD‐DLT as the preoperative level.
Conclusion
The liver of patients with MSUD can be used safely without concern regarding long‐term complications or de novo MSUD development. LD‐DLT using the MSUD liver can expand the donor pool as an alternative graft in pediatric LT.
The aim of this case series study is to illustrate clinicopathological features of MPV17-related mitochondrial DNA depletion syndrome (MDDS). Mitochondrial respiratory chain disorders (MRCDs) are rare conditions with various clinical manifestations that depend on the genetic type, with subsequent severity of liver disease. Approximately 40% of patients present with clinical symptoms in the neonatal period, whereas the remaining 60% of patients have a more delayed onset of their disease. Clinical presentations include acute liver failure (ALF) or chronic liver failure with liver cirrhosis, which are cruelly progressive and fatal. Among subclassified MRCDs, MDDS is a group of genetically heterogeneous diseases that can cause ALF in 10% to 20% of affected children, which could be an indication for liver transplantation (LT). (1) Neonatal ALF is a serious disorder with a variety of potential causes with generally unsatisfactory long-term prognosis. Because of the high risk of hidden and progressive extrahepatic (particularly neuromuscular and cardiopulmonary) manifestations, combined with the difficulty to accurately identify the etiology in a timely manner, the indications for performing LT in such patients with MDDS remain controversial.The protein MPV17 is found in the inner mitochondrial membrane, and its absence causes failure of oxidative phosphorylation and depletion of mitochondrial DNA. MPV17-related hepatocerebral MDDS is characterized by hepatic encephalopathy and liver failure with a poor prognosis. LT has been performed in 20% of affected children with MDDS; however, the outcomes were not satisfactory because of progressive extrahepatic manifestations. (2) It is therefore imperative to have a rational approach to define the appropriate criteria of the indications for LT in children affected by MPV17-related MDDS. Although MPV17-related mitochondrial DNA maintenance defects are known for having extreme clinical, biochemical, and genetical heterogeneity, there is pathognomonic evidence that implies the specific diagnosis prior to LT.
Single‐incision laparoscopic repair of a congenital Morgagni diaphragmatic hernia using a suture‐assisting needle was performed in a 1‐year‐old boy. Three ports were inserted through a single umbilical incision to repair the 2.5 × 2.3‐cm defect. The full‐thickness muscle layer of the anterior abdominal wall and the posterior rim of the defect were penetrated with the suture‐assisting needle holding a thread, which was then released. The needle tip was pulled back over the muscle layer, shifted laterally, and again passed through the muscle layer and the posterior rim. The thread was then captured by the needle and pulled out through the anterior abdominal wall. Five mattress sutures were placed in this way and tied subcutaneously. The postoperative course was uneventful, and the cosmetic outcome was favorable. A suture‐assisting needle is useful for completing full‐thickness anterior abdominal wall repair, which is important for preventing the recurrence of a congenital Morgagni diaphragmatic hernia.
Background: LT is an elective treatment choice for children diagnosed with GSD1b that can improve their quality of life and stabilize their glucose intolerance. However, careful attention should be paid to immunosuppression after LT due to the susceptibility to infection because of neutropenia and neutrophil dysfunction in GSD1b patients. This study revealed the immunological features and complications in the early post-LT period.
Methods:We compared findings between 11 (1.9%) children with GSD1b and 273 children with BA. Analyses using the PSM were performed to overcome selection bias.Results: Despite persistent low tacrolimus trough levels in GSD1b patients, none of these children developed TCMR within 1 month after LDLT (GSD1b: 0/11 [0%] vs. BA: 86/273 [31.5%], p = .038). This result was also confirmed in PSM. The incidence of bloodstream infections was higher in GSD1b patients than in BA patients in the early phase of the post-transplant period (GSD1b: 4/11 [36.4%] vs. BA: 33/273 [12.1%], p = .041), but not reach statistical significance in PSM. In a phenotypic analysis, the ratio of CD8 + T cells in GSD1b recipients' peripheral blood mononuclear cell samples was lower than in recipients with BA through the first month after LDLT.
Conclusions:We found that GSD1b recipients were more likely to develop postoperative bloodstream infection than recipients with BA but did not experience TCMR despite low tacrolimus levels in the early post-LDLT period. A tailored immunosuppression protocol should be prepared for GSD1b recipients after LDLT.
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